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Cardiac manifestations in the antiphospholipid syndrome
Felicia Tenedios1, Doruk Erkan, Michael D Lockshin
1Hospital for Special Surgery, Department of Rheumatology, Weill Medical College of Cornell University, 535 East 70(th) Street, New York, NY 10021, USA. tenediosf@hss.edu
Insights
Antiphospholipid antibodies (aPL) are not just markers but may cause thrombosis and pregnancy issues in antiphospholipid syndrome (APS). Research explores aPL
Area of Science:
- Rheumatology and Immunology
- Cardiovascular Medicine
- Hematology
Background:
- Antiphospholipid antibodies (aPL) are recognized markers for antiphospholipid syndrome (APS).
- Emerging evidence suggests aPL may actively contribute to the pathogenesis of APS complications.
- The mechanisms underlying aPL pathogenicity are complex and multifaceted.
Purpose of the Study:
- To elucidate the pathogenic mechanisms of aPL in APS.
- To detail the cardiac manifestations associated with APS.
- To investigate the role of aPL in vascular thrombosis and pregnancy morbidity.
Main Methods:
- Review of experimental evidence on aPL function.
- Analysis of molecular pathways involved in aPL pathogenicity.
- Correlation of aPL presence with clinical manifestations in APS.
Main Results:
- aPL contribute to thrombosis via endothelial activation, protein C pathway inhibition, platelet dysfunction, and complement activation.
- Cardiac manifestations in APS include valve abnormalities, atherosclerosis, myocardial infarction, and pulmonary hypertension.
- Valve disease is the most frequent cardiac complication, though its precise mechanism remains unclear.
Conclusions:
- aPL play a causative role in APS, driving thrombosis and pregnancy morbidity.
- aPL induce prothrombotic changes in the vasculature.
- Cardiac involvement, particularly valve disease, is a significant feature of APS, warranting further investigation into its pathogenesis.
Abstract:
Experimental evidence reveals that aPL are not only markers of APS, but also may play a causative role in the development of vascular thrombosis and pregnancy morbidity. The pathogenic mechanisms of aPL seem to be heterogeneous, including endothelial cell activation, the direct inhibition of the activated protein C pathway, abnormalities in platelet function, and in complement activation. aPLs induce proadhesive, proinflammatory, and procoagulant molecules that provide a persuasive explanation for induction of thrombosis in APS. Cardiac manifestations in APS include valve abnormalities (valve thickening and vegetations), occlusive arterial disease (atherosclerosis and myocardial infarction), intracardiac emboli, ventricular dysfunction, and pulmonary hypertension. aPL may be associated with accelerated atherosclerosis in APS patients. Valve disease is the most important and most common cardiac manifestation of APS. The precise mechanism by which valves become deformed is not yet fully known.
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